Abstract
Background: Autoimmune progesterone dermatitis (APD) is a rare skin condition caused by sensitivity to high levels of progesterone secreted during the luteal phase of the menstrual cycle. This may be due to various pathophysiological mechanisms including a Type I and Type IV hypersensitivity reaction, and potential cross-reactivity with other endogenous steroid hormones such as 17-α-hydroxyprogesterone. We present the case of a patient with APD who had her flare-up episodes controlled using omalizumab, after a bilateral oophorectomy failed to resolve her symptoms. Clinical Case: A 34-year-old female presented to our Endocrine Clinic with marked Cushingoid features secondary to high-dose oral prednisone prescribed for APD diagnosed six years earlier. She first developed a pruritic maculopapular rash on her arms and legs just after the birth of her second child in 2009. The rash was also associated with headaches and diffuse angioedema. It presented in a cyclical fashion, beginning one to two days before the start of her menstrual cycle, and ending shortly after it was complete. The severity of symptoms increased as time went on, and flare-ups began to also include dyspnea, nausea, vomiting and abdominal pain. After three years of persistent symptoms, the diagnosis of APD was confirmed by a progesterone skin test. Her symptoms were improved with oral prednisone use, however breakthrough episodes still occurred. After multiple failed treatment modalities, she elected bilateral oophorectomy in 2018. However, her symptoms of APD persisted and she still required high-dose oral prednisone. Her condition was further complicated by vasomotor menopausal symptoms and progressive iatrogenic Cushing’s syndrome. She eventually was started on Omalizumab, which helped resolve her APD symptoms and allowed her to wean off prednisone. Vasomotor menopausal symptoms were resolved using conjugated estrogens with bazedoxifene. However, her symptoms of diffuse bony indeterminate bony pain and arthralgias which started whilst on prednisone have persisted in spite of discontinuing prednisone. Conclusion: To our knowledge, this is only the third case of APD which was successfully treated with Omalizumab and the first case where a bilateral oophorectomy failed to resolve symptoms of APD in the literature. Our case also demonstrates the complications of vasomotor menopause symptoms secondary to a bilateral oophorectomy, as well as the adverse effects of long-term glucocorticoid therapy.
Facial cleft? The first case of manitoba‐oculo‐tricho‐anal syndrome with novel mutations in China: a case report